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Pulmonary hypertension in systemic lupus erythematosus
Benjamin E Schreiber1, Michelle J Connolly, J Gerry Coghlan
1National Pulmonary Hypertension Service, Royal Free Hospital NHS Foundation Trust, UK.
Best Practice & Research. Clinical Rheumatology
|November 19, 2013
Summary
Systemic lupus erythematosus (SLE) can cause pulmonary arterial hypertension (PAH). While treatments exist, immunosuppression may offer unique benefits for PAH in lupus patients, though it remains a fatal condition.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Systemic lupus erythematosus (SLE) is linked to various pulmonary hypertension (PH) forms.
- These include pulmonary thromboembolic disease, left heart disease, lung disease, and isolated pulmonary arterial hypertension (PAH).
Observation:
- The exact prevalence of PAH in SLE patients is unknown, estimated at no more than 1%.
- Current treatments for lupus-associated PAH mirror those for other PAH causes, utilizing pulmonary vasodilators like phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and prostacyclin analogues.
Findings:
- Case series suggest a potential specialized role for immunosuppression in treating lupus-associated PAH.
- The prognosis for lupus-associated PAH is more favorable than for systemic sclerosis-associated PAH.
Implications:
- Lupus-associated PAH, while having a better prognosis than some forms, remains a predominantly fatal condition.
- Further research into the role of immunosuppression in lupus-associated PAH is warranted.
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